-
1
-
-
0024424270
-
Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
-
Riordan J.R., Rommens J.M., Kerem B.S., et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science. 245:1989;1066-1073.
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.S.3
-
2
-
-
0024453308
-
Identification of the cystic fibrosis gene: Chromosome walking and jumping
-
Rommens J.M., Iannuzzi M.C., Kerem B.S., et al. Identification of the cystic fibrosis gene: chromosome walking and jumping. Science. 245:1989;1059-1065.
-
(1989)
Science
, vol.245
, pp. 1059-1065
-
-
Rommens, J.M.1
Iannuzzi, M.C.2
Kerem, B.S.3
-
3
-
-
0025760318
-
Genomic DNA sequence of the cystic fibrosis transmembrane conductance regulator
-
Zielinski J., Rozmahel R., Bozon D., et al. Genomic DNA sequence of the cystic fibrosis transmembrane conductance regulator. Genomics. 10:1991;241-248.
-
(1991)
Genomics
, vol.10
, pp. 241-248
-
-
Zielinski, J.1
Rozmahel, R.2
Bozon, D.3
-
4
-
-
0026523829
-
Cystic fibrosis: Molecular biology and therapeutic implications
-
Collins F.S. Cystic fibrosis: molecular biology and therapeutic implications. Science. 256:1992;774-779.
-
(1992)
Science
, vol.256
, pp. 774-779
-
-
Collins, F.S.1
-
6
-
-
0036120193
-
The cystic fibrosis transmembrane conductance regulator: An intriguing protein with pleiotropic functions
-
Vankeerberghen A., Cuppens H., Cassiman J.J. The cystic fibrosis transmembrane conductance regulator: an intriguing protein with pleiotropic functions. J Cystic Fibrosis. 1:2002;13-29.
-
(2002)
J Cystic Fibrosis
, vol.1
, pp. 13-29
-
-
Vankeerberghen, A.1
Cuppens, H.2
Cassiman, J.J.3
-
7
-
-
0027517995
-
Correlation between genotype and phenotype in cystic fibrosis
-
Correlation between genotype and phenotype in cystic fibrosis. N Engl J Med. 329:1993;1308-1313.
-
(1993)
N Engl J Med
, vol.329
, pp. 1308-1313
-
-
-
8
-
-
0028229223
-
The origin of the major cystic fibrosis mutation (delta F508) in European populations
-
Morral N., Bertranpetit J., Estivill X., et al. The origin of the major cystic fibrosis mutation (delta F508) in European populations. Nat Genet. 7:1994;169-175.
-
(1994)
Nat Genet
, vol.7
, pp. 169-175
-
-
Morral, N.1
Bertranpetit, J.2
Estivill, X.3
-
9
-
-
0028177981
-
Delta F508 genotype does not predict disease severity in an ethnically diverse cystic fibrosis population
-
Lester L.A., Kraut J., Lloyd-Still J., et al. Delta F508 genotype does not predict disease severity in an ethnically diverse cystic fibrosis population. Pediatrics. 93:1994;114-118.
-
(1994)
Pediatrics
, vol.93
, pp. 114-118
-
-
Lester, L.A.1
Kraut, J.2
Lloyd-Still, J.3
-
10
-
-
0035213684
-
Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings
-
Bronsveld I., Mekus F., Bijman J., et al. Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings. J Clin Invest. 108:2001;1705-1715.
-
(2001)
J Clin Invest
, vol.108
, pp. 1705-1715
-
-
Bronsveld, I.1
Mekus, F.2
Bijman, J.3
-
11
-
-
0032935226
-
HLA class II polymorphism in cystic fibrosis: A possible modifier of pulmonary phenotype
-
Aron Y., Polla B.S., Bienvenu T., et al. HLA class II polymorphism in cystic fibrosis: a possible modifier of pulmonary phenotype. Am J Respir Crit Care Med. 159:1999;1464-1468.
-
(1999)
Am J Respir Crit Care Med
, vol.159
, pp. 1464-1468
-
-
Aron, Y.1
Polla, B.S.2
Bienvenu, T.3
-
12
-
-
0034523682
-
Airway NO levels in CF patients are related to a polymorphism in the neuronal NO synthase (NOS1) gene
-
Grasemann H., Knauer N., Eijmes J., et al. Airway NO levels in CF patients are related to a polymorphism in the neuronal NO synthase (NOS1) gene. Am J Respir Crit Care Med. 162:2000;2172-2176.
-
(2000)
Am J Respir Crit Care Med
, vol.162
, pp. 2172-2176
-
-
Grasemann, H.1
Knauer, N.2
Eijmes, J.3
-
13
-
-
0028473632
-
Allotypes of alpha1-antitrypsin in cystic fibrosis patients homozygous and heterozygous for delta F508
-
Döring G., Krogh-Johansen H., Weidinger S., et al. Allotypes of alpha1-antitrypsin in cystic fibrosis patients homozygous and heterozygous for delta F508. Pediatr Pulmonol. 18:1994;3-7.
-
(1994)
Pediatr Pulmonol
, vol.18
, pp. 3-7
-
-
Döring, G.1
Krogh-Johansen, H.2
Weidinger, S.3
-
14
-
-
0035153395
-
Association of alpha(1)-antichymotrypsin deficiency with milder lung disease in patients with cystic fibrosis
-
Mahadeva R., Sharples L., Ross-Russell R.I., et al. Association of alpha(1)-antichymotrypsin deficiency with milder lung disease in patients with cystic fibrosis. Thorax. 56:2001;53-58.
-
(2001)
Thorax
, vol.56
, pp. 53-58
-
-
Mahadeva, R.1
Sharples, L.2
Ross-Russell, R.I.3
-
15
-
-
0032695668
-
Association of mannose-binding lectin gene heterogeneity with severity of lung disease and survival in cystic fibrosis
-
Garred P., Pressler T., Madsen H.O., et al. Association of mannose-binding lectin gene heterogeneity with severity of lung disease and survival in cystic fibrosis. J Clin Invest. 104:1999;431-437.
-
(1999)
J Clin Invest
, vol.104
, pp. 431-437
-
-
Garred, P.1
Pressler, T.2
Madsen, H.O.3
-
16
-
-
0036020992
-
2-adrenoreceptor polymorphisms as modifiers of lung disease in cystic fibrosis
-
2-adrenoreceptor polymorphisms as modifiers of lung disease in cystic fibrosis. Pharmacogenetics. 12:2002;347-353.
-
(2002)
Pharmacogenetics
, vol.12
, pp. 347-353
-
-
Büscher, R.1
Eilmes, K.J.2
Grasemann, H.3
-
17
-
-
0033028607
-
Detection of a cystic fibrosis modifier locus for meconium ileus on human chromosome 19q13
-
Zielenski J., Corey M., Rozmahel R., et al. Detection of a cystic fibrosis modifier locus for meconium ileus on human chromosome 19q13. Nat Genet. 22:1999;128-129.
-
(1999)
Nat Genet
, vol.22
, pp. 128-129
-
-
Zielenski, J.1
Corey, M.2
Rozmahel, R.3
-
18
-
-
0023789980
-
Evidence for reduced Cl- and increased Na+ permeability in cystic fibrosis human primary cell cultures
-
Boucher R., Cotton C.U., Gatzy J.T., et al. Evidence for reduced Cl- and increased Na+ permeability in cystic fibrosis human primary cell cultures. J Physiol. 404:1988;77-103.
-
(1988)
J Physiol
, vol.404
, pp. 77-103
-
-
Boucher, R.1
Cotton, C.U.2
Gatzy, J.T.3
-
19
-
-
0032478144
-
Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator
-
Schwiebert E.M., Morales M.M., Devidas S., et al. Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator. Proc Natl Acad Sci USA. 95:1998;2674-2689.
-
(1998)
Proc Natl Acad Sci USA
, vol.95
, pp. 2674-2689
-
-
Schwiebert, E.M.1
Morales, M.M.2
Devidas, S.3
-
20
-
-
0033581885
-
Activation of the epithelial Na+ channel (ENaC) requires CFTR Cl- channel function
-
Reddy M.M., Light M.J., Quinton P.M. Activation of the epithelial Na+ channel (ENaC) requires CFTR Cl- channel function. Nature. 402:1999;301-304.
-
(1999)
Nature
, vol.402
, pp. 301-304
-
-
Reddy, M.M.1
Light, M.J.2
Quinton, P.M.3
-
21
-
-
0025874599
-
Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis
-
Knowles M.R., Clarke L.L., Boucher R.C. Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis. N Engl J Med. 325:1991;533-538.
-
(1991)
N Engl J Med
, vol.325
, pp. 533-538
-
-
Knowles, M.R.1
Clarke, L.L.2
Boucher, R.C.3
-
24
-
-
0028914085
-
Early pulmonary inflammation in infants with cystic fibrosis
-
Khan T.Z., Wagener J.S., Bost T., et al. Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med. 151:1995;1075-1082.
-
(1995)
Am J Respir Crit Care Med
, vol.151
, pp. 1075-1082
-
-
Khan, T.Z.1
Wagener, J.S.2
Bost, T.3
-
25
-
-
0030909647
-
Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice
-
Zahm J.M., Gaillard D., Dupuit F., et al. Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice. Am J Physiol. 272:1997;C853-C859.
-
(1997)
Am J Physiol
, vol.272
-
-
Zahm, J.M.1
Gaillard, D.2
Dupuit, F.3
-
26
-
-
0033844485
-
Inflammation and infection in naive human cystic fibrosis airway grafts
-
Tirouvanziam R., de Bentzmann S., Hubeau C., et al. Inflammation and infection in naive human cystic fibrosis airway grafts. Am J Respir Cell Mol Biol. 23:2000;121-127.
-
(2000)
Am J Respir Cell Mol Biol
, vol.23
, pp. 121-127
-
-
Tirouvanziam, R.1
De Bentzmann, S.2
Hubeau, C.3
-
27
-
-
0030768159
-
Lower airway inflammation in infants and young children with cystic fibrosis
-
Armstrong D.S., Grimwood K., Carlin J.B., et al. Lower airway inflammation in infants and young children with cystic fibrosis. Am J Respir Crit Care Med. 156:1997;1197-1204.
-
(1997)
Am J Respir Crit Care Med
, vol.156
, pp. 1197-1204
-
-
Armstrong, D.S.1
Grimwood, K.2
Carlin, J.B.3
-
28
-
-
0029372481
-
Normal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine interleukin-10, which is downregulated in cystic fibrosis
-
Bonfield T.L., Konstan M.W., Burfeind P., et al. Normal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine interleukin-10, which is downregulated in cystic fibrosis. Am J Respir Cell Mol Biol. 13:1995;257-261.
-
(1995)
Am J Respir Cell Mol Biol
, vol.13
, pp. 257-261
-
-
Bonfield, T.L.1
Konstan, M.W.2
Burfeind, P.3
-
29
-
-
0033456217
-
IL-10 attenuates excessive inflammation in chronic Pseudomonas infection in mice
-
Chmiel J.F., Konstan M.W., Knesebeck J.E., et al. IL-10 attenuates excessive inflammation in chronic Pseudomonas infection in mice. Am J Respir Crit Care Med. 160:1999;2040-2047.
-
(1999)
Am J Respir Crit Care Med
, vol.160
, pp. 2040-2047
-
-
Chmiel, J.F.1
Konstan, M.W.2
Knesebeck, J.E.3
-
30
-
-
0035923599
-
Molecular basis for defective glycosylation and Pseudomonas pathogenesis in cystic fibrosis lung
-
Poschet J.F., Boucher J.C., Tatterson L., et al. Molecular basis for defective glycosylation and Pseudomonas pathogenesis in cystic fibrosis lung. Proc Natl Acad Sci USA. 98:2001;13972-13977.
-
(2001)
Proc Natl Acad Sci USA
, vol.98
, pp. 13972-13977
-
-
Poschet, J.F.1
Boucher, J.C.2
Tatterson, L.3
-
31
-
-
0028920643
-
Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface
-
Imundo L., Barasch J., Prince A., et al. Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface. Proc Natl Acad Sci USA. 92:1995;3019-3023.
-
(1995)
Proc Natl Acad Sci USA
, vol.92
, pp. 3019-3023
-
-
Imundo, L.1
Barasch, J.2
Prince, A.3
-
32
-
-
0030021470
-
Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections
-
Pier G.B., Grout M., Zaidi T.S., et al. Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections. Science. 271:1996;64-67.
-
(1996)
Science
, vol.271
, pp. 64-67
-
-
Pier, G.B.1
Grout, M.2
Zaidi, T.S.3
-
33
-
-
0029870085
-
Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid
-
Smith J.J., Travis S.M., Greenberg E.P., et al. Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell. 85:1996;229-236.
-
(1996)
Cell
, vol.85
, pp. 229-236
-
-
Smith, J.J.1
Travis, S.M.2
Greenberg, E.P.3
-
34
-
-
0035178729
-
Altered NaCl concentration of airway surface liquid in cystic fibrosis
-
Widdicombe J.H. Altered NaCl concentration of airway surface liquid in cystic fibrosis. Pflügers Arch Eur J Physiol. 443:(suppl 1):2001;S8-S10.
-
(2001)
Pflügers Arch Eur J Physiol
, vol.443
, Issue.SUPPL. 1
-
-
Widdicombe, J.H.1
-
35
-
-
0033560645
-
Transfer of a cathelicidin peptide antibiotic gene restores bacterial killing in a cystic fibrosis xenograft model
-
Bals R., Weiner D.J., Meegalla R.L., et al. Transfer of a cathelicidin peptide antibiotic gene restores bacterial killing in a cystic fibrosis xenograft model. J Clin Invest. 103:1999;1113-1117.
-
(1999)
J Clin Invest
, vol.103
, pp. 1113-1117
-
-
Bals, R.1
Weiner, D.J.2
Meegalla, R.L.3
-
36
-
-
0035132659
-
Noninvasive in vivo fluorescence measurement of airway-surface liquid depth, salt concentration, and pH
-
Jayaraman S., Song Y., Vetrivel L., et al. Noninvasive in vivo fluorescence measurement of airway-surface liquid depth, salt concentration, and pH. J Clin Invest. 107:2001;317-324.
-
(2001)
J Clin Invest
, vol.107
, pp. 317-324
-
-
Jayaraman, S.1
Song, Y.2
Vetrivel, L.3
-
37
-
-
0032433707
-
Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease
-
Matsui H., Grubb B.R., Tarran R., et al. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell. 95:1998;1005-1015.
-
(1998)
Cell
, vol.95
, pp. 1005-1015
-
-
Matsui, H.1
Grubb, B.R.2
Tarran, R.3
-
38
-
-
0036167851
-
Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients
-
Worlitzsch D., Tarran R., Ulrich M., et al. Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients. J Clin Invest. 109:2002;317-325.
-
(2002)
J Clin Invest
, vol.109
, pp. 317-325
-
-
Worlitzsch, D.1
Tarran, R.2
Ulrich, M.3
-
39
-
-
0033056054
-
Mucoid conversion of Pseudomonas aeruginosa by hydrogen peroxide: A mechanism for virulence activation in the cystic fibrosis lung
-
Mathee K., Ciofu O., Sternberg C., et al. Mucoid conversion of Pseudomonas aeruginosa by hydrogen peroxide: a mechanism for virulence activation in the cystic fibrosis lung. Microbiology. 145:1999;1349-1357.
-
(1999)
Microbiology
, vol.145
, pp. 1349-1357
-
-
Mathee, K.1
Ciofu, O.2
Sternberg, C.3
-
40
-
-
0028015883
-
Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation
-
Konstan M.W., Hilliard K.A., Norvell T.M., et al. Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation. Am J Respir Crit Care Med. 150:1994;448-454.
-
(1994)
Am J Respir Crit Care Med
, vol.150
, pp. 448-454
-
-
Konstan, M.W.1
Hilliard, K.A.2
Norvell, T.M.3
-
41
-
-
0021917723
-
Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis
-
Kopelman H., Durie P., Gaskin K., et al. Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis. N Engl J Med. 312:1985;329-334.
-
(1985)
N Engl J Med
, vol.312
, pp. 329-334
-
-
Kopelman, H.1
Durie, P.2
Gaskin, K.3
-
42
-
-
0025720681
-
The cystic fibrosis gene and resting energy expenditure
-
Fried M.D., Durie P.R., Tsui L.C., et al. The cystic fibrosis gene and resting energy expenditure. J Pediatr. 119:1991;913-916.
-
(1991)
J Pediatr
, vol.119
, pp. 913-916
-
-
Fried, M.D.1
Durie, P.R.2
Tsui, L.C.3
-
43
-
-
0028314401
-
Diabetes mellitus in Danish cystic fibrosis patients: Prevalence and late diabetic complications
-
Lanng S., Thornsteinsson B., Lund-Anderen C., et al. Diabetes mellitus in Danish cystic fibrosis patients: prevalence and late diabetic complications. Acta Paediatr. 83:1994;72-77.
-
(1994)
Acta Paediatr
, vol.83
, pp. 72-77
-
-
Lanng, S.1
Thornsteinsson, B.2
Lund-Anderen, C.3
-
44
-
-
0030760747
-
Insulin resistance with altered secretory kinetics and reduced proinsulin in cystic fibrosis patients
-
Holl R.W., Wolf A., Thon A., et al. Insulin resistance with altered secretory kinetics and reduced proinsulin in cystic fibrosis patients. J Pediatr Gastroenterol Nutr. 25:1997;188-193.
-
(1997)
J Pediatr Gastroenterol Nutr
, vol.25
, pp. 188-193
-
-
Holl, R.W.1
Wolf, A.2
Thon, A.3
-
45
-
-
0029097769
-
Male fertility in cystic fibrosis
-
Dodge J.A. Male fertility in cystic fibrosis. Lancet. 346:1995;587-588.
-
(1995)
Lancet
, vol.346
, pp. 587-588
-
-
Dodge, J.A.1
-
46
-
-
0015885904
-
Water and electrolytes in cervical mucus from patients with cystic fibrosis
-
Kopito L.E., Kosasky H.J., Shwachman H. Water and electrolytes in cervical mucus from patients with cystic fibrosis. Fertil Steril. 24:1973;512-516.
-
(1973)
Fertil Steril
, vol.24
, pp. 512-516
-
-
Kopito, L.E.1
Kosasky, H.J.2
Shwachman, H.3
-
47
-
-
0031900652
-
The diagnosis of cystic fibrosis: A consensus statement
-
Rosenstein B.J., Cutting G.R. The diagnosis of cystic fibrosis: a consensus statement. J Pediatr. 132:1998;589-595.
-
(1998)
J Pediatr
, vol.132
, pp. 589-595
-
-
Rosenstein, B.J.1
Cutting, G.R.2
-
48
-
-
0031016388
-
Current concepts: The diagnosis of cystic fibrosis
-
Stern R.C. Current concepts: the diagnosis of cystic fibrosis. N Engl J Med. 336:1997;487-491.
-
(1997)
N Engl J Med
, vol.336
, pp. 487-491
-
-
Stern, R.C.1
-
49
-
-
0031900650
-
Making the diagnosis of cystic fibrosis
-
Wilmott R.W. Making the diagnosis of cystic fibrosis. J Pediatr. 132:1998;563-565.
-
(1998)
J Pediatr
, vol.132
, pp. 563-565
-
-
Wilmott, R.W.1
-
50
-
-
0025772974
-
Ion transport abnormalities in rectal suction biopsies from children with cystic fibrosis
-
Veeze H.J., Sinaasappel M., Bijman J., et al. Ion transport abnormalities in rectal suction biopsies from children with cystic fibrosis. Gastroenterology. 101:1991;398-3403.
-
(1991)
Gastroenterology
, vol.101
, pp. 398-3403
-
-
Veeze, H.J.1
Sinaasappel, M.2
Bijman, J.3
-
51
-
-
0033866283
-
Time to think again: Cystic fibrosis is not an "all or none" disease
-
Bush A., Wallis C. Time to think again: cystic fibrosis is not an "all or none" disease. Pediatr Pulmonol. 30:2000;139-144.
-
(2000)
Pediatr Pulmonol
, vol.30
, pp. 139-144
-
-
Bush, A.1
Wallis, C.2
-
52
-
-
0013983024
-
Stool trypsin and chymotrypsin: Value in the diagnosis of pancreatic insufficiency in cystic fibrosis
-
Barbero G.J., Sibinga M.S., Marino J.M. Stool trypsin and chymotrypsin: value in the diagnosis of pancreatic insufficiency in cystic fibrosis. Am J Dis Child. 112:1966;536-540.
-
(1966)
Am J Dis Child
, vol.112
, pp. 536-540
-
-
Barbero, G.J.1
Sibinga, M.S.2
Marino, J.M.3
-
53
-
-
0032737592
-
Faecal elastase 1: A marker of exocrine pancreatic insufficiency in cystic fibrosis
-
Phillips I.J., Rowe D.J., Dewar P., et al. Faecal elastase 1: a marker of exocrine pancreatic insufficiency in cystic fibrosis. Ann Clin Biochem. 36:1999;739-742.
-
(1999)
Ann Clin Biochem
, vol.36
, pp. 739-742
-
-
Phillips, I.J.1
Rowe, D.J.2
Dewar, P.3
-
54
-
-
0000518360
-
Paranasal sinuses in cystic fibrosis: Incidence of roentgen abnormalities
-
Gharib R., Allen R.P., Joos H.A., et al. Paranasal sinuses in cystic fibrosis: incidence of roentgen abnormalities. Am J Dis Child. 108:1964;499-502.
-
(1964)
Am J Dis Child
, vol.108
, pp. 499-502
-
-
Gharib, R.1
Allen, R.P.2
Joos, H.A.3
-
55
-
-
1842339924
-
Distinct spectrum of CFTR mutations in congenital absence of vas deferens
-
Dörk T., Dworniczak B., Aulehla-Scholz C., et al. Distinct spectrum of CFTR mutations in congenital absence of vas deferens. Hum Genet. 100:1997;365-377.
-
(1997)
Hum Genet
, vol.100
, pp. 365-377
-
-
Dörk, T.1
Dworniczak, B.2
Aulehla-Scholz, C.3
-
57
-
-
0034596493
-
Neonatal screening for cystic fibrosis in Brittany, France: Assessment of 10 years' experience and impact on prenatal diagnosis
-
Scotet V., de Braekeleer M., Roussey M., et al. Neonatal screening for cystic fibrosis in Brittany, France: assessment of 10 years' experience and impact on prenatal diagnosis. Lancet. 356:2000;789-794.
-
(2000)
Lancet
, vol.356
, pp. 789-794
-
-
Scotet, V.1
De Braekeleer, M.2
Roussey, M.3
-
58
-
-
0030803131
-
Nutritional benefits of neonatal screening for cystic fibrosis
-
Farrell P.M., Kosorok M.R., Laxova A., et al. Nutritional benefits of neonatal screening for cystic fibrosis. N Engl J Med. 337:1997;963-969.
-
(1997)
N Engl J Med
, vol.337
, pp. 963-969
-
-
Farrell, P.M.1
Kosorok, M.R.2
Laxova, A.3
-
59
-
-
17744372496
-
Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth
-
Farrell P.M., Kosorok M.R., Rock M.J., et al. Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth. Pediatrics. 107:2001;1-13.
-
(2001)
Pediatrics
, vol.107
, pp. 1-13
-
-
Farrell, P.M.1
Kosorok, M.R.2
Rock, M.J.3
-
60
-
-
0029833357
-
Nutrition and survival in cystic fibrosis
-
Elborn J.S., Bell S.C. Nutrition and survival in cystic fibrosis. Thorax. 51:1996;971-972.
-
(1996)
Thorax
, vol.51
, pp. 971-972
-
-
Elborn, J.S.1
Bell, S.C.2
-
61
-
-
0031267463
-
Acquisition of Pseudomonas aeruginosa in children with cystic fibrosis
-
Farrell P.M., Shen G., Splaingard M., et al. Acquisition of Pseudomonas aeruginosa in children with cystic fibrosis. Pediatrics. 100:1997;E2.
-
(1997)
Pediatrics
, vol.100
, pp. 2
-
-
Farrell, P.M.1
Shen, G.2
Splaingard, M.3
-
62
-
-
0034834535
-
Gene therapy in cystic fibrosis
-
Flotte T.R., Laube B.L. Gene therapy in cystic fibrosis. Chest. 120:2001;124S-131S.
-
(2001)
Chest
, vol.120
-
-
Flotte, T.R.1
Laube, B.L.2
-
63
-
-
0029100239
-
A controlled study of adenoviral-vector-mediated gene transfer in the nasal epithelium of patients with cystic fibrosis
-
Knowles M.R., Hohneker K.W., Zhou Z., et al. A controlled study of adenoviral-vector-mediated gene transfer in the nasal epithelium of patients with cystic fibrosis. N Engl J Med. 333:1995;823-831.
-
(1995)
N Engl J Med
, vol.333
, pp. 823-831
-
-
Knowles, M.R.1
Hohneker, K.W.2
Zhou, Z.3
-
64
-
-
0033586375
-
Cationic lipid-mediated CFTR gene transfer to the lungs and nose of patients with cystic fibrosis: A double-blind placebo-controlled trial
-
Alton E.W., Stern M., Farley R., et al. Cationic lipid-mediated CFTR gene transfer to the lungs and nose of patients with cystic fibrosis: a double-blind placebo-controlled trial. Lancet. 353:1999;947-954.
-
(1999)
Lancet
, vol.353
, pp. 947-954
-
-
Alton, E.W.1
Stern, M.2
Farley, R.3
-
65
-
-
13344286312
-
Repeat administration of an adenovirus vector encoding cystic fibrosis transmembrane conductance regulator to the nasal epithelium of patients with cystic fibrosis
-
Zabner J., Ramsey B.W., Meeker D.P., et al. Repeat administration of an adenovirus vector encoding cystic fibrosis transmembrane conductance regulator to the nasal epithelium of patients with cystic fibrosis. J Clin Invest. 97:1996;1504-1511.
-
(1996)
J Clin Invest
, vol.97
, pp. 1504-1511
-
-
Zabner, J.1
Ramsey, B.W.2
Meeker, D.P.3
-
66
-
-
0035894583
-
Gentamicin in pharmocogenetic approach to treatment of cystic fibrosis
-
Hamilton J.W. Gentamicin in pharmocogenetic approach to treatment of cystic fibrosis. Lancet. 358:2001;2014-2016.
-
(2001)
Lancet
, vol.358
, pp. 2014-2016
-
-
Hamilton, J.W.1
-
67
-
-
0025242929
-
Defective intracellular traffic and processing of CFTR is the molecular basis of most cystic fibrosis
-
Cheng S.H., Gregory R.J., Marshall J., et al. Defective intracellular traffic and processing of CFTR is the molecular basis of most cystic fibrosis. Cell. 63:1990;827-834.
-
(1990)
Cell
, vol.63
, pp. 827-834
-
-
Cheng, S.H.1
Gregory, R.J.2
Marshall, J.3
-
68
-
-
0031915434
-
Limited proteolysis as a probe for arrested conformational maturation of delta F508 CFTR
-
Zhang F., Kartner N., Lukacs G.L. Limited proteolysis as a probe for arrested conformational maturation of delta F508 CFTR. Nat Struct Biol. 5:1998;180-183.
-
(1998)
Nat Struct Biol
, vol.5
, pp. 180-183
-
-
Zhang, F.1
Kartner, N.2
Lukacs, G.L.3
-
69
-
-
0032588980
-
DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis
-
Kalin N., Claass A., Sommer M., et al. DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis. J Clin Invest. 103:1999;1379-1389.
-
(1999)
J Clin Invest
, vol.103
, pp. 1379-1389
-
-
Kalin, N.1
Claass, A.2
Sommer, M.3
-
70
-
-
0033918281
-
Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells
-
Penque D., Mendes F., Beck S., et al. Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells. Lab Invest. 80:2000;857-868.
-
(2000)
Lab Invest
, vol.80
, pp. 857-868
-
-
Penque, D.1
Mendes, F.2
Beck, S.3
-
71
-
-
0033625415
-
Activation of deltaF508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX
-
Andersson C., Roomans G.M. Activation of deltaF508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX. Eur Respir J. 15:2000;937-941.
-
(2000)
Eur Respir J
, vol.15
, pp. 937-941
-
-
Andersson, C.1
Roomans, G.M.2
-
72
-
-
0031889082
-
A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: Partial restoration of nasal epithelial CFTR function
-
Rubenstein R.C., Zeitlin P.L. A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function. Am J Respir Crit Care Med. 157:1998;484-490.
-
(1998)
Am J Respir Crit Care Med
, vol.157
, pp. 484-490
-
-
Rubenstein, R.C.1
Zeitlin, P.L.2
-
73
-
-
0036161237
-
A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis
-
McCarty N.A., Standaert T.A., Teresi M., et al. A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis. Pediatr Pulmonol. 33:2002;90-98.
-
(2002)
Pediatr Pulmonol
, vol.33
, pp. 90-98
-
-
McCarty, N.A.1
Standaert, T.A.2
Teresi, M.3
-
74
-
-
0035200620
-
Cell-based assay for high-throughput quantitative screening of CFTR chloride transport agonists
-
Galietta L.V., Jayaraman S., Verkman A.S. Cell-based assay for high-throughput quantitative screening of CFTR chloride transport agonists. Am J Physiol Cell Physiol. 281:2001;C1734-C1742.
-
(2001)
Am J Physiol Cell Physiol
, vol.281
-
-
Galietta, L.V.1
Jayaraman, S.2
Verkman, A.S.3
-
75
-
-
0034192547
-
Deletion of phenylalanine 508 causes attenuated phosphorylation-dependent activation of CFTR chloride channels
-
Wang F., Zeltwanger S., Hu S., et al. Deletion of phenylalanine 508 causes attenuated phosphorylation-dependent activation of CFTR chloride channels. J Physiol. 524:2000;637-648.
-
(2000)
J Physiol
, vol.524
, pp. 637-648
-
-
Wang, F.1
Zeltwanger, S.2
Hu, S.3
-
76
-
-
0025236867
-
A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis
-
Knowles M.R., Church N.L., Waltner W.E., et al. A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis. N Engl J Med. 322:1990;1189-1194.
-
(1990)
N Engl J Med
, vol.322
, pp. 1189-1194
-
-
Knowles, M.R.1
Church, N.L.2
Waltner, W.E.3
-
77
-
-
0025874599
-
Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis
-
Knowles M.R., Clarke L.L., Boucher R.C. Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis. N Engl J Med. 325:1991;533-538.
-
(1991)
N Engl J Med
, vol.325
, pp. 533-538
-
-
Knowles, M.R.1
Clarke, L.L.2
Boucher, R.C.3
-
78
-
-
0032833866
-
The effect of topical benzamil and amiloride on nasal potential difference in cystic fibrosis
-
Rodgers H.C., Knox A.J. The effect of topical benzamil and amiloride on nasal potential difference in cystic fibrosis. Eur Respir J. 14:1999;693-696.
-
(1999)
Eur Respir J
, vol.14
, pp. 693-696
-
-
Rodgers, H.C.1
Knox, A.J.2
-
79
-
-
0034896551
-
Safety of aerosolized INS 365 in patients with mild to moderate cystic fibrosis: Results of a phase I multi-center study
-
Noone P.G., Hamblett N., Accurso F., et al. Safety of aerosolized INS 365 in patients with mild to moderate cystic fibrosis: results of a phase I multi-center study. Pediatr Pulmonol. 32:2001;122-128.
-
(2001)
Pediatr Pulmonol
, vol.32
, pp. 122-128
-
-
Noone, P.G.1
Hamblett, N.2
Accurso, F.3
-
80
-
-
0013359613
-
Management of infection in cystic fibrosis
-
Döring G. Management of infection in cystic fibrosis. Hosp Pharm. 5:2002;65-69.
-
(2002)
Hosp Pharm
, vol.5
, pp. 65-69
-
-
Döring, G.1
-
81
-
-
0025077322
-
Pseudomonas aeruginosa infection in cystic fibrosis and its management
-
Høiby N., Koch C. Pseudomonas aeruginosa infection in cystic fibrosis and its management. Thorax. 45:1990;881-884.
-
(1990)
Thorax
, vol.45
, pp. 881-884
-
-
Høiby, N.1
Koch, C.2
-
82
-
-
0027395872
-
The changing epidemiology of cystic fibrosis
-
Fitzsimmons F.C. The changing epidemiology of cystic fibrosis. J Pediatr. 122:1993;1-9.
-
(1993)
J Pediatr
, vol.122
, pp. 1-9
-
-
Fitzsimmons, F.C.1
-
83
-
-
0019961926
-
Antibiotic treatment of Staphylococcus aureus infection in cystic fibrosis
-
Szaff M., Hoiby N. Antibiotic treatment of Staphylococcus aureus infection in cystic fibrosis. Acta Paediatr Scand. 71:1982;21-26.
-
(1982)
Acta Paediatr Scand
, vol.71
, pp. 21-26
-
-
Szaff, M.1
Hoiby, N.2
-
84
-
-
0031958323
-
Persistent infection with small colony variant strains of Staphylococcus aureus in patients with cystic fibrosis
-
Kahl B., Herrmann M., Everding A.S., et al. Persistent infection with small colony variant strains of Staphylococcus aureus in patients with cystic fibrosis. J Infect Dis. 177:1998;1023-1029.
-
(1998)
J Infect Dis
, vol.177
, pp. 1023-1029
-
-
Kahl, B.1
Herrmann, M.2
Everding, A.S.3
-
85
-
-
0028350689
-
Prognosis in cystic fibrosis treated with continuous flucloxacillin from the neonatal period
-
Weaver L.T., Green M.R., Nicholson K., et al. Prognosis in cystic fibrosis treated with continuous flucloxacillin from the neonatal period. Arch Dis Child. 70:1994;84-89.
-
(1994)
Arch Dis Child
, vol.70
, pp. 84-89
-
-
Weaver, L.T.1
Green, M.R.2
Nicholson, K.3
-
86
-
-
0035125425
-
Effect of continuous anti-staphylococcal therapy on the rate of P aeruginosa acquisition in patients with cystic fibrosis
-
Ratjen F., Comes G., Paul K., et al. Effect of continuous anti-staphylococcal therapy on the rate of P aeruginosa acquisition in patients with cystic fibrosis. Pediatr Pulmonol. 31:2001;13-16.
-
(2001)
Pediatr Pulmonol
, vol.31
, pp. 13-16
-
-
Ratjen, F.1
Comes, G.2
Paul, K.3
-
87
-
-
0036220978
-
Antibiotic prophylaxis in infants and young children with cystic fibrosis: A randomized controlled trial
-
Stutman H.R., Lieberman J.M., Nussbaum E., et al. Antibiotic prophylaxis in infants and young children with cystic fibrosis: a randomized controlled trial. J Pediatr. 140:2002;299-305.
-
(2002)
J Pediatr
, vol.140
, pp. 299-305
-
-
Stutman, H.R.1
Lieberman, J.M.2
Nussbaum, E.3
-
88
-
-
84921429624
-
Prophylactic antibiotics for cystic fibrosis (Cochrane Review)
-
Smyth A., Walters S. Prophylactic antibiotics for cystic fibrosis (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD001021.
-
(2000)
Cochrane Database Syst Rev
, vol.2
, pp. 001021
-
-
Smyth, A.1
Walters, S.2
-
89
-
-
0034685940
-
High frequency of hypermutable Pseudomonas aeruginosa in cystic fibrosis lung infection
-
Oliver A., Canton R., Campo P., et al. High frequency of hypermutable Pseudomonas aeruginosa in cystic fibrosis lung infection. Science. 288:2000;1251-1254.
-
(2000)
Science
, vol.288
, pp. 1251-1254
-
-
Oliver, A.1
Canton, R.2
Campo, P.3
-
90
-
-
0033773012
-
Antibiotic therapy against Pseudomonas aeruginosa in cystic fibrosis: A European consensus
-
Döring G., Conway S.P., Heijerman H.G., et al. Antibiotic therapy against Pseudomonas aeruginosa in cystic fibrosis: a European consensus. Eur Respir J. 16:2000;749-767.
-
(2000)
Eur Respir J
, vol.16
, pp. 749-767
-
-
Döring, G.1
Conway, S.P.2
Heijerman, H.G.3
-
91
-
-
0029781290
-
Adaptive resistance to tobramycin in Pseudomonas aeruginosa lung infection in cystic fibrosis
-
Barclay M.L., Begg E.J., Chambers S.T., et al. Adaptive resistance to tobramycin in Pseudomonas aeruginosa lung infection in cystic fibrosis. J Antimicrob Chemother. 37:1996;1155-1164.
-
(1996)
J Antimicrob Chemother
, vol.37
, pp. 1155-1164
-
-
Barclay, M.L.1
Begg, E.J.2
Chambers, S.T.3
-
92
-
-
0035226425
-
Elective versus symptomatic intravenous antibiotic therapy for cystic fibrosis
-
Breen L., Aswani N. Elective versus symptomatic intravenous antibiotic therapy for cystic fibrosis. Cochrane Database Syst Rev. 4:2001;CD002767.
-
(2001)
Cochrane Database Syst Rev
, vol.4
, pp. 002767
-
-
Breen, L.1
Aswani, N.2
-
93
-
-
0029921514
-
Improved survival in the Danish center-treated cystic fibrosis patients: Results of aggressive treatment
-
Frederiksen B., Lanng S., Koch C., et al. Improved survival in the Danish center-treated cystic fibrosis patients: results of aggressive treatment. Pediatr Pulmonol. 21:1996;153.
-
(1996)
Pediatr Pulmonol
, vol.21
, pp. 153
-
-
Frederiksen, B.1
Lanng, S.2
Koch, C.3
-
94
-
-
0034107646
-
Elective versus symptomatic antibiotic treatment in cystic fibrosis patients with chronic Pseudomonas infection of the lungs
-
Elborn J.S., Prescott R.J., Stack B.H., et al. Elective versus symptomatic antibiotic treatment in cystic fibrosis patients with chronic Pseudomonas infection of the lungs. Thorax. 55:2000;355-358.
-
(2000)
Thorax
, vol.55
, pp. 355-358
-
-
Elborn, J.S.1
Prescott, R.J.2
Stack, B.H.3
-
95
-
-
0024488435
-
Long-term tobramycin inhalation in cystic fibrosis
-
Steinkamp G., Tümmler H., Gappa M., et al. Long-term tobramycin inhalation in cystic fibrosis. Pediatr Pulmonol. 6:1989;91-98.
-
(1989)
Pediatr Pulmonol
, vol.6
, pp. 91-98
-
-
Steinkamp, G.1
Tümmler, H.2
Gappa, M.3
-
96
-
-
0023265976
-
Colistin inhalation therapy in cystic fibrosis patients with chronic P aeruginosa infection
-
Jensen T., Pedersen S.S., Garne S., et al. Colistin inhalation therapy in cystic fibrosis patients with chronic P aeruginosa infection. J Antimicrob Chemother. 19:1987;831-838.
-
(1987)
J Antimicrob Chemother
, vol.19
, pp. 831-838
-
-
Jensen, T.1
Pedersen, S.S.2
Garne, S.3
-
97
-
-
0033531143
-
Intermittent administration of inhaled tobramycin in patients with cystic fibrosis
-
Ramsey B.W., Pepe M.S., Quan J.M., et al. Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. N Engl J Med. 340:1999;23-30.
-
(1999)
N Engl J Med
, vol.340
, pp. 23-30
-
-
Ramsey, B.W.1
Pepe, M.S.2
Quan, J.M.3
-
98
-
-
84921431098
-
Nebulised anti-pseudomonal antibiotics for cystic fibrosis (Cochrane Review)
-
Ryan G., Mukhopadhyay S., Singh M. Nebulised anti-pseudomonal antibiotics for cystic fibrosis (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD001021.
-
(2000)
Cochrane Database Syst Rev
, vol.2
, pp. 001021
-
-
Ryan, G.1
Mukhopadhyay, S.2
Singh, M.3
-
99
-
-
0031807730
-
Improvement of survival in patients with diffuse panbronchiolitis treated with low-dose erythromycin
-
Kudoh S., Azuma A., Yamamoto M., et al. Improvement of survival in patients with diffuse panbronchiolitis treated with low-dose erythromycin. Am J Respir Crit Care Med. 157:1998;1829-1832.
-
(1998)
Am J Respir Crit Care Med
, vol.157
, pp. 1829-1832
-
-
Kudoh, S.1
Azuma, A.2
Yamamoto, M.3
-
100
-
-
0344624839
-
Long-term azithromycin may improve lung function in children with cystic fibrosis
-
Jaffe A., Francis J., Rosenthal M., et al. Long-term azithromycin may improve lung function in children with cystic fibrosis. Lancet. 351:1998;420.
-
(1998)
Lancet
, vol.351
, pp. 420
-
-
Jaffe, A.1
Francis, J.2
Rosenthal, M.3
-
101
-
-
0036185807
-
Effect of long term treatment with azithromycin on disease parameters in cystic fibrosis: A randomised trial
-
Wolter J., Seeney S., Bell S., et al. Effect of long term treatment with azithromycin on disease parameters in cystic fibrosis: a randomised trial. Thorax. 57:2002;212-216.
-
(2002)
Thorax
, vol.57
, pp. 212-216
-
-
Wolter, J.1
Seeney, S.2
Bell, S.3
-
102
-
-
0037190575
-
Long term azithromycin in children with cystic fibrosis: A randomised, placebo-controlled crossover trial
-
Equi A., Balfour-Lynn I., Bush A., Rosenthal M. Long term azithromycin in children with cystic fibrosis: a randomised, placebo-controlled crossover trial. Lancet. 360:2002;978-984.
-
(2002)
Lancet
, vol.360
, pp. 978-984
-
-
Equi, A.1
Balfour-Lynn, I.2
Bush, A.3
Rosenthal, M.4
-
104
-
-
0021996644
-
Nebulised colomycin for early Pseudomonas colonisation in cystic fibrosis
-
Littlewood J.M., Miller M.G., Ghoneim A.T., et al. Nebulised colomycin for early Pseudomonas colonisation in cystic fibrosis. Lancet. 1:1985;865.
-
(1985)
Lancet
, vol.1
, pp. 865
-
-
Littlewood, J.M.1
Miller, M.G.2
Ghoneim, A.T.3
-
105
-
-
0025868478
-
Prevention of chronic Pseudomonas aeruginosa colonisation by early treatment
-
Valerius N.H., Koch C., Hoiby N. Prevention of chronic Pseudomonas aeruginosa colonisation by early treatment. Lancet. 338:1991;725-726.
-
(1991)
Lancet
, vol.338
, pp. 725-726
-
-
Valerius, N.H.1
Koch, C.2
Hoiby, N.3
-
106
-
-
2642712510
-
Placebo-controlled double-blind randomized study of aerosolized tobramycin for early treatment of Pseudomonas aeruginosa colonisation in cystic fibrosis
-
Wiesemann H.G., Steinkamp G., Ratjen F., et al. Placebo-controlled double-blind randomized study of aerosolized tobramycin for early treatment of Pseudomonas aeruginosa colonisation in cystic fibrosis. Pediatr Pulmonol. 26:1998;88-92.
-
(1998)
Pediatr Pulmonol
, vol.26
, pp. 88-92
-
-
Wiesemann, H.G.1
Steinkamp, G.2
Ratjen, F.3
-
107
-
-
0035934545
-
Eradication of Pseudomonas aeruginosa with inhaled tobramycin in patients with cystic fibrosis
-
Ratjen F., Döring G., Nikolaizik W. Eradication of Pseudomonas aeruginosa with inhaled tobramycin in patients with cystic fibrosis. Lancet. 358:2001;983-984.
-
(2001)
Lancet
, vol.358
, pp. 983-984
-
-
Ratjen, F.1
Döring, G.2
Nikolaizik, W.3
-
108
-
-
0021970474
-
Alternate-day prednisone reduces morbitity and improves pulmonary function in cystic fibrosis
-
Auerbach H.S., Willaims M., Kirkpatrick J.A., et al. Alternate-day prednisone reduces morbitity and improves pulmonary function in cystic fibrosis. Lancet. 2:1985;686-688.
-
(1985)
Lancet
, vol.2
, pp. 686-688
-
-
Auerbach, H.S.1
Willaims, M.2
Kirkpatrick, J.A.3
-
109
-
-
0028969594
-
A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis
-
Eigen H., Rosenstein B.J., Fitzsimmons S., et al. A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. J Pediatr. 126:1995;515-523.
-
(1995)
J Pediatr
, vol.126
, pp. 515-523
-
-
Eigen, H.1
Rosenstein, B.J.2
Fitzsimmons, S.3
-
110
-
-
0034704799
-
FitzSimmons SC, Allen DB, et al. Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis
-
Lai H.C. FitzSimmons SC, Allen DB, et al. Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis. N Engl J Med. 342:2000;851-859.
-
(2000)
N Engl J Med
, vol.342
, pp. 851-859
-
-
Lai, H.C.1
-
113
-
-
0028914667
-
Effect of high-dose ibuprofen in patients with cystic fibrosis
-
Konstan M.W., Byard P.J., Hoppel C.L., et al. Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med. 332:1995;848-854.
-
(1995)
N Engl J Med
, vol.332
, pp. 848-854
-
-
Konstan, M.W.1
Byard, P.J.2
Hoppel, C.L.3
-
114
-
-
0000879923
-
Immunology of cystic fibrosis
-
M.E. Hodson, & D. Geddes. London: Arnold
-
Döring G., Knight R., Bellon G. Immunology of cystic fibrosis. Hodson M.E., Geddes D. Cystic fibrosis. 2000;109-141 Arnold, London.
-
(2000)
Cystic fibrosis
, pp. 109-141
-
-
Döring, G.1
Knight, R.2
Bellon, G.3
-
115
-
-
0026474443
-
Modulation of airway inflammation in cystic fibrosis: In vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitor
-
McElvaney N.G., Nakamura H., Birrer P., et al. Modulation of airway inflammation in cystic fibrosis: in vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitor. J Clin Invest. 90:1992;1296-1301.
-
(1992)
J Clin Invest
, vol.90
, pp. 1296-1301
-
-
McElvaney, N.G.1
Nakamura, H.2
Birrer, P.3
-
116
-
-
0027495712
-
Pharmacokinetics of recombinant secretory leukoprotease inhibitor aerosolized to normals and individuals with cystic fibrosis
-
McElvaney N.G., Doujaiji B., Moan M.J., et al. Pharmacokinetics of recombinant secretory leukoprotease inhibitor aerosolized to normals and individuals with cystic fibrosis. Am Rev Respir Dis. 148:1993;1056-1060.
-
(1993)
Am Rev Respir Dis
, vol.148
, pp. 1056-1060
-
-
McElvaney, N.G.1
Doujaiji, B.2
Moan, M.J.3
-
118
-
-
0032882814
-
Aerosolized prolastin suppresses bacterial proliferation in a model of chronic Pseudomonas aeruginosa lung infection
-
Cantin A.M., Woods D.E. Aerosolized prolastin suppresses bacterial proliferation in a model of chronic Pseudomonas aeruginosa lung infection. Am J Respir Crit Care Med. 160:1999;1130-1135.
-
(1999)
Am J Respir Crit Care Med
, vol.160
, pp. 1130-1135
-
-
Cantin, A.M.1
Woods, D.E.2
-
119
-
-
0022378715
-
A double-blind placebo controlled trial with oral ambroxol and n-acetylcysteine for mucolytic treatment in cystic fibrosis
-
Ratjen F., Wönne R., Posselt H.G., et al. A double-blind placebo controlled trial with oral ambroxol and n-acetylcysteine for mucolytic treatment in cystic fibrosis. Eur J Pediatr. 144:1985;374-378.
-
(1985)
Eur J Pediatr
, vol.144
, pp. 374-378
-
-
Ratjen, F.1
Wönne, R.2
Posselt, H.G.3
-
120
-
-
0028129568
-
Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis
-
Fuchs H.J., Borowitz D.S., Christiansen D.H., et al. Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. N Engl J Med. 331:1994;637-642.
-
(1994)
N Engl J Med
, vol.331
, pp. 637-642
-
-
Fuchs, H.J.1
Borowitz, D.S.2
Christiansen, D.H.3
-
121
-
-
0035666058
-
A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities
-
Quan J.M., Tiddens H.A., Sy J.P., et al. A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities. J Pediatr. 139:2001;813-820.
-
(2001)
J Pediatr
, vol.139
, pp. 813-820
-
-
Quan, J.M.1
Tiddens, H.A.2
Sy, J.P.3
-
122
-
-
84921431060
-
Deoxyribonuclease for cystic fibrosis (Cochrane Review)
-
Kearney C.E., Wallis C.E. Deoxyribonuclease for cystic fibrosis (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD001127.
-
(2000)
Cochrane Database Syst Rev
, vol.2
, pp. 001127
-
-
Kearney, C.E.1
Wallis, C.E.2
-
123
-
-
0035922668
-
Comparison of hypertonic saline and alternate-day or daily recombinant human deoxyribonuclease in children with cystic fibrosis: A randomised trial
-
Suri R., Metcalfe C., Lees B., et al. Comparison of hypertonic saline and alternate-day or daily recombinant human deoxyribonuclease in children with cystic fibrosis: a randomised trial. Lancet. 358:2001;1316-1321.
-
(2001)
Lancet
, vol.358
, pp. 1316-1321
-
-
Suri, R.1
Metcalfe, C.2
Lees, B.3
-
124
-
-
84921430394
-
Nebulised hypertonic saline for cystic fibrosis (Cochrane Review)
-
Wark P.A., McDonald V. Nebulised hypertonic saline for cystic fibrosis (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD001506.
-
(2000)
Cochrane Database Syst Rev
, vol.2
, pp. 001506
-
-
Wark, P.A.1
McDonald, V.2
-
125
-
-
0034570842
-
Worldwide thoracic organ transplantation: A report from the UNOS/ISHLT international Registry for Thoracic Organ Transplantation
-
Bennet L.E., Keck B.M., Daily O.P., et al. Worldwide thoracic organ transplantation: a report from the UNOS/ISHLT international Registry for Thoracic Organ Transplantation. Clin Transpl. 1:2000;31-44.
-
(2000)
Clin Transpl
, vol.1
, pp. 31-44
-
-
Bennet, L.E.1
Keck, B.M.2
Daily, O.P.3
-
126
-
-
0035814353
-
Survival effect of lung transplantation among patients with cystic fibrosis
-
Liou T.G., Adler F.R., Cahill B.C., et al. Survival effect of lung transplantation among patients with cystic fibrosis. JAMA. 286:2001;2683-2689.
-
(2001)
JAMA
, vol.286
, pp. 2683-2689
-
-
Liou, T.G.1
Adler, F.R.2
Cahill, B.C.3
-
127
-
-
0033530379
-
Lung transplantation and life extension in children with cystic fibrosis
-
Aurora P., Whitehead B., Wade A., et al. Lung transplantation and life extension in children with cystic fibrosis. Lancet. 354:1999;1591-1593.
-
(1999)
Lancet
, vol.354
, pp. 1591-1593
-
-
Aurora, P.1
Whitehead, B.2
Wade, A.3
-
128
-
-
0035659459
-
Lung transplantation for cystic fibrosis patients with Burkholderia cepacia complex: Survival linked to genomovar type
-
Aris R.M., Routh J.C., LiPuma J.J., et al. Lung transplantation for cystic fibrosis patients with Burkholderia cepacia complex: survival linked to genomovar type. Am J Respir Crit Care Med. 164:2001;2102-2106.
-
(2001)
Am J Respir Crit Care Med
, vol.164
, pp. 2102-2106
-
-
Aris, R.M.1
Routh, J.C.2
LiPuma, J.J.3
-
129
-
-
0026520359
-
Prediction of mortality in patients with cystic fibrosis
-
Kerem E., Reisman J., Corey M., et al. Prediction of mortality in patients with cystic fibrosis. N Engl J Med. 326:1992;1187-1191.
-
(1992)
N Engl J Med
, vol.326
, pp. 1187-1191
-
-
Kerem, E.1
Reisman, J.2
Corey, M.3
-
130
-
-
0035053808
-
Living donor lung transplantation
-
Cohen R.G., Starnes V.A. Living donor lung transplantation. World J Surg. 25:2001;244-250.
-
(2001)
World J Surg
, vol.25
, pp. 244-250
-
-
Cohen, R.G.1
Starnes, V.A.2
-
131
-
-
0020070795
-
Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption
-
Gaskin K., Gurwitz D., Durie P., et al. Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption. J Pediatr. 100:1982;857-862.
-
(1982)
J Pediatr
, vol.100
, pp. 857-862
-
-
Gaskin, K.1
Gurwitz, D.2
Durie, P.3
-
132
-
-
0023923679
-
A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto
-
Corey M., McLaughlin F.J., Williams M., et al. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol. 41:1988;583-591.
-
(1988)
J Clin Epidemiol
, vol.41
, pp. 583-591
-
-
Corey, M.1
McLaughlin, F.J.2
Williams, M.3
-
133
-
-
0036299705
-
Nutrition in patients with cystic fibrosis: A European consensus
-
Sinaasappel M., Stern M., Littlewood J., et al. Nutrition in patients with cystic fibrosis: a European consensus. J Cystic Fibrosis. 1:2002;51-75.
-
(2002)
J Cystic Fibrosis
, vol.1
, pp. 51-75
-
-
Sinaasappel, M.1
Stern, M.2
Littlewood, J.3
-
134
-
-
84921430676
-
Oral calorie supplements for cystic fibrosis (Cochrane Review)
-
Smyth R., Walters S. Oral calorie supplements for cystic fibrosis (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD000406.
-
(2000)
Cochrane Database Syst Rev
, vol.2
, pp. 000406
-
-
Smyth, R.1
Walters, S.2
-
135
-
-
44949089717
-
Enteral tube feeding for cystic fibrosis (Cochrane Review)
-
Conway S.P., Morton A., Wolfe S. Enteral tube feeding for cystic fibrosis (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD001198.
-
(2000)
Cochrane Database Syst Rev
, vol.2
, pp. 001198
-
-
Conway, S.P.1
Morton, A.2
Wolfe, S.3
-
136
-
-
84921431266
-
Ursodeoxycholic acid for cystic fibrosis-related liver disease (Cochrane Review)
-
Cheng K., Ashby D., Smyth R. Ursodeoxycholic acid for cystic fibrosis-related liver disease (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD000222.
-
(2000)
Cochrane Database Syst Rev
, vol.2
, pp. 000222
-
-
Cheng, K.1
Ashby, D.2
Smyth, R.3
-
137
-
-
0036230252
-
Transplantation for cystic fibrosis: Outcome following early liver transplantation
-
Milkiewicz P., Skiba G., Kelly D., et al. Transplantation for cystic fibrosis: outcome following early liver transplantation. J Gastroenterol Hepatol. 17:2002;208-213.
-
(2002)
J Gastroenterol Hepatol
, vol.17
, pp. 208-213
-
-
Milkiewicz, P.1
Skiba, G.2
Kelly, D.3
-
138
-
-
0034046150
-
International comparison of median age at death from cystic fibrosis
-
Fogarthy A., Hubbard R., Britton J. International comparison of median age at death from cystic fibrosis. Chest. 117:2000;1656-1660.
-
(2000)
Chest
, vol.117
, pp. 1656-1660
-
-
Fogarthy, A.1
Hubbard, R.2
Britton, J.3
-
140
-
-
0034987271
-
The association of socioeconomic status with outcomes in cystic fibrosis patients in the United States
-
Schechter M.S., Shelton B.J., Margolis P.A., et al. The association of socioeconomic status with outcomes in cystic fibrosis patients in the United States. Am J Respir Crit Care Med. 163:2001;1331-1337.
-
(2001)
Am J Respir Crit Care Med
, vol.163
, pp. 1331-1337
-
-
Schechter, M.S.1
Shelton, B.J.2
Margolis, P.A.3
|